Table of Contents
Definition / general | Epidemiology | Sites | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosisCite this page: Mannan R, Yuan S. Low grade nasopharyngeal papillary adenocarcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalpapillaryadenocarcinoma.html. Accessed March 24th, 2023.
Definition / general
- Low grade primary adenocarcinoma of nasopharynx, derived from surface epithelium
Epidemiology
- Uncommon tumor of nasopharynx
- Median age 37 years but occurs over a wide age range
- Not associated with wood dust exposure or other known factors
- Not related to EBV
- No gender predilection
Sites
- May occur anywhere in nasopharynx but preferred locations are roof, posterior wall and lateral wall
Clinical features
- Nasal obstruction is most common presenting symptom
- Tumors usually remain confined within nasopharynx
- Excellent prognosis
- Slow growing and indolent
- Rarely recurs (if incomplete excision)
- No metastases reported to date
Case reports
- 39 year old woman with biphasic low grade nasopharyngeal papillary adenocarcinoma with a prominent spindle cell component (Head Neck Pathol 2011;5:306)
- Association with Turner syndrome (Med J Malaysia 1998;53:104)
Treatment
- Complete surgical excision is usually curative
Gross description
- Soft, exophytic mass with papillary appearance
- Size can vary from a few millimeters to 3.0 cm
Microscopic (histologic) description
- Infiltrative tumor with papillary and glandular growth patterns
- Papillary structures are complex with arborization and fibrovascular cores
- Glandular growth has cribriform or back to back glands
- Lined by cuboidal or columnar cells with pink cytoplasm and round / oval nuclei that are variably clear or hyperchromatic
- Mild to moderate nuclear pleomorphism, no / rare nucleoli
- Mitotic figures are inconspicuous
- Occasionally psammoma bodies may be present
- May be focal necrosis
- No angiolymphatic invasion or perineural invasion
Microscopic (histologic) images
Positive stains
- PAS (intracytoplasmic, diastase resistant granules), mucin (intracellular or luminal)
- Cytokeratin (diffuse), EMA (diffuse), CEA (focal) (Am J Surg Pathol 1988;12:946)
- TTF1 (J Formos Med Assoc 2015;114:473)
Negative stains
Differential diagnosis
- Intestinal type adenocarcinoma - papillary type: nasal cavity and paranasal sinuses, usually wood dust exposure, more papillary and less glandular, tall columnar and goblet cells, "dirty" background with hemorrhage and inflammation
- Low grade papillary adenocarcinoma of salivary gland origin: rare in nasopharynx, arises from minor salivary glands in submucosa, not surface epithelium, usually S100+, aggressive with local recurrence (27%) and nodal metastases (17%)
- Papillary thyroid carcinoma: thyroglobulin+, no epithelial dysplasia; note: TTF1 may be positive in both
- Papilloma of surface epithelium or minor salivary gland origin